Pleuropulmonary blastoma

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Historia

Thirty months old young girl with large right chest mass.

Wyniki

There is a large mass occupying most of the right chest cavity and displacing mediastinal structures to the left. There is heterogeneous FDG avidity depending of the lesion’s density. Cystic components are hypoactive while solid parts are highly FDG-avid. SUV max is 9.9 in the posteromedial aspect of the lesion.

No other suspicious lesion.

DDx

Pleuropulmonary blastoma

Neuroblastoma

Ewing’s sarcoma

PNET

Rhabdomysarcoma

Diagnoza

Pleuropulmonary blastoma

Dyskusja

Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignant neoplasm occuring in early childhood, most cases occuring before six years old. This tumour is associated with mutation of the DICER1 gene.

There are three types of PPB. Type I is purely cystic. Type II is composed of cystic and solid areas, whereas type III is a solid tumor without cystic spaces. Type I tumors occur in younger children than type II/III, with a median age around 1 year old. It is also known that type I can progress to more aggressive type II or III. The type of PPB is a strong predictor of outcome. Type I has the better prognosis and type III the worst. The presence of distant metastasis at diagnosis is another poor prognostic factor.

Naffaa et al. described CT findings in four patients with PPB. They reported large masses with complete or near-complete opacification of the hemithorax and heterogeneous low attenuation. There was associated pleural effusion but absence of chest wall invasion. All tumors were on the right side. In another study, Priest et al. reported that 70% of PPBs were located in the right hemithorax.

To our knowledge, there is only one report of FDG PET/CT in the context of PPB. Geiger et al. used the FDG PET/CT to assess response to neoadjuvant chemotherapy and decide the timing of surgery, facilitating tumor resection.